Functional impact of augmentative and alternative communication scale: development of an outcome measure for educators of students with complex communication needs.

PURPOSE: The study aim was to develop and assess the measurement properties of the Functional Impact of Augmentative and Alternative Communication - Educator (FIAAC-E) scale - an educator-reported measure designed to detect change in factors associated with communicative participation in students who use augmentative and alternative communication (AAC). METHODS: The study had three phases. In phase 1 (content validity), 22 content specialists independently rated the relevancy of 22 dimensions proposed for the new scale. In phase 2 (item generation, face validity), 8 educators independently completed a preliminary version of the FIAAC-E scale and flagged items that were unclear. Next, these educators adjusted the wording of unclear items during consensus-building sessions. In phase 3 (item reduction, reliability, convergent validity), 62 educators completed an online survey that included the revised FIAAC-E scale. Survey data were used to shorten the scale, estimate its reliability, and evaluate support for its convergent validity. RESULTS: In phase 1, the 11 dimensions ranked most highly by content specialists were selected for inclusion in the preliminary scale. In phase 2, educators reviewed 113 items and revised 33 to improve clarity. In phase 3, correlational statistics informed the selection of 77 items for the shortened scale. Subsequent data analyses indicated acceptable levels of internal consistency and test-retest reliability and support for convergent validity of the preliminary measure. CONCLUSION: The study provides emerging evidence that supports the FIAAC-E scale as a reliable way to evaluate communicative participation in children and youth who use AAC systems at school.Implications for rehabilitationUsing a reliable means to measure the effectiveness of AAC for children and youth at school may reveal important factors that influence successful communicative, academic, and social participation in school.The FIAAC-E scale is a promising educator-reported questionnaire to inform the development of communication goals and monitor progress towards meeting these goals for students with complex communication needs.

Evaluation of the multidimensional effects of adaptive seating interventions for young children with non-ambulatory cerebral palsy.

PURPOSE: To investigate the short-term effectiveness of the first adaptive seating system received by children with non-ambulatory cerebral palsy (CP) who are classified as level IV or V according to the Gross Motor Function Classification System. MATERIALS AND METHODS: A trained clinical assessor examined 20 children with non-ambulatory CP (mean age: 4.5 years) for their trunk control ability in static, active, and reactive tasks using the Segmental Assessment of Trunk Control. Their primary caregivers were also interviewed about their child's activity and participation using the Paediatric Evaluation of Disability Inventory Computer - Adaptive Test in daily activity and social/cognitive domains and Family Impact of Assistive Technology Scale for Adaptive Seating in child and family functioning domains. Data for each measure were collected 3 times: at baseline (pre-intervention) and then 6 weeks and 3 months after children received their first adaptive seating system. RESULTS AND CONCLUSION: The static and active trunk control scores between baseline and 6 weeks, and baseline and 3 months significantly improved. Daily activity scaled scores significantly improved between baseline and 3 months, and 6 weeks and 3 months. Significant, large gains in child and family functioning overall were detected between baseline and 6 weeks, and baseline and 3 months. These findings provide emerging evidence of multidimensional effects associated with the introduction of a first adaptive seating system into the lives of young children with non-ambulatory CPIMPLICATIONS FOR REHABILITATIONThe introduction of an adaptive seating system into the wheelchair of children with non-ambulatory cerebral palsy may be associated with short-term gains in body function, activities, participation and aspects of the child's environment.

Endarterectomy of carotid artery bifurcation in the setting of a persistent hypoglossal artery and anomalous collateral vascular supply.

Presented is a patient with carotid artery stenosis resulting in crescendo anterior and posterior circulation transient ischemic attacks. Treatment was complicated by a rare persistent hypoglossal artery (HGA) arising from the left internal carotid artery in addition to severe contralateral carotid disease, hypoplastic vertebral arteries, and incomplete circle of Willis. A carotid endarterectomy with shunting was performed, maintaining perfusion of both the proper left internal carotid artery and HGA. This is a rare case of carotid stenosis in the setting of a persistent HGA with contralateral carotid disease and highlights the importance of planning intracranial perfusion before carotid surgery.

A Novel Recurrent COL5A1 Genetic Variant Is Associated With a Dysplasia-Associated Arterial Disease Exhibiting Dissections and Fibromuscular Dysplasia.

OBJECTIVE: While rare variants in the COL5A1 gene have been associated with classical Ehlers-Danlos syndrome and rarely with arterial dissections, recurrent variants in COL5A1 underlying a systemic arteriopathy have not been described. Monogenic forms of multifocal fibromuscular dysplasia (mFMD) have not been previously defined. Approach and Results: We studied 4 independent probands with the COL5A1 pathogenic variant c.1540G>A, p.(Gly514Ser) who presented with arterial aneurysms, dissections, tortuosity, and mFMD affecting multiple arteries. Arterial medial fibroplasia and smooth muscle cell disorganization were confirmed histologically. The COL5A1 c.1540G>A variant is predicted to be pathogenic in silico and absent in gnomAD. The c.1540G>A variant is on a shared 160.1 kb haplotype with 0.4% frequency in Europeans. Furthermore, exome sequencing data from a cohort of 264 individuals with mFMD were examined for COL5A1 variants. In this mFMD cohort, COL5A1 c.1540G>A and 6 additional relatively rare COL5A1 variants predicted to be deleterious in silico were identified and were associated with arterial dissections (P=0.005). CONCLUSIONS: COL5A1 c.1540G>A is the first recurring variant recognized to be associated with arterial dissections and mFMD. This variant presents with a phenotype reminiscent of vascular Ehlers-Danlos syndrome. A shared haplotype among probands supports the existence of a common founder. Relatively rare COL5A1 genetic variants predicted to be deleterious by in silico analysis were identified in ≈2.7% of mFMD cases, and as they were enriched in patients with arterial dissections, may act as disease modifiers. Molecular testing for COL5A1 should be considered in patients with a phenotype overlapping with vascular Ehlers-Danlos syndrome and mFMD.

Gas in the inferior vena cava from severe emphysematous pyelonephritis.

Emphysematous pyelonephritis is a life-threatening, urologic emergency. We present the case of a 54-yearold female who was found to have a large volume of air in her renal vein and inferior vena cava from severe emphysematous pyelonephritis. In this case report, the medical and operative management of this patient are reviewed, and the literature on the management of emphysematous pyelonephritis is summarized.

Renovascular hypertension from the BCR-ABL tyrosine kinase inhibitor ponatinib.

Drug-induced hypertension is one of the commonest causes of secondary hypertension. In the last few years, secondary hypertension due to tyrosine kinase inhibitors, from the vascular endothelial growth factor class, has been recognized to be an important cause of hypertension, as well as proteinuria, and occasionally kidney dysfunction in some cases. Less well-recognized is that BCR-ABL tyrosine kinase inhibitors also have adverse vascular effects. These manifest as vascular stenoses in large vessels, which may sometimes cause renal artery stenosis and subsequent hypertension. We describe a case report which presented as classical bilateral renal artery stenosis, and responded to revascularization. Increased awareness of these effects, as well as research into the pathogenesis, may provide more insight into vascular biology.

Toward greater involvement of youth with complex communication needs in the selection of augmentative and alternative communication devices.

Purpose: The study aim was to examine the measurement properties of the Youth Evaluation of Products Scale for Augmentative and Alternative Communication (YEP-AAC) as a means to understand the product attitudes of youth toward their AAC devices.Methods: In study 1, four males and two females between the ages of 8 and 17 years (median age = 14 years) participated in face-to-face interviews. A researcher administered the YEP-AAC and then asked youths to explain the rationale for their item ratings. Two researchers analyzed masked transcripts of the interviews to predict the valence of item ratings assigned by youths. In study 2, 18 families participated in a mailed survey to examine the reliability and validity of the scale domains. Seventeen mothers/one father and their children (9 male/9 female) between the ages of 10 and 20 years participated (median age = 16 years).Results: The level of chance-corrected agreement between valences converted from youth ratings on the YEP-AAC and those assigned by researchers was fair (weighted kappa = 0.38). Internal consistencies of the scale domains were acceptable. Youths who rated their AAC devices more favourably had higher functional communication as hypothesized.Conclusion: The two studies provide support for the face validity, internal consistency and convergent validity of the YEP-AAC as a measure of the product attitudes of youth with complex communication needs.Implications for rehabilitationDeveloping reliable ways to understand the product needs, preferences and values of youth with complex communication needs will help to support the selection, acquisition and use of augmentative and alternative communication (AAC) devices.Consumer socialization helps to explain how youth may acquire the skills, knowledge and experience relevant to their role as AAC consumers.Reliable youth-report questionnaires that are grounded in consumer socialization provide an expedient way to understand the product attitudes of young consumers with complex communication needs.

Virtual community centre for power wheelchair training: Experience of children and clinicians.

PURPOSE: To: 1) characterize the overall experience in using the McGill immersive wheelchair - community centre (miWe-CC) simulator; and 2) investigate the experience of presence (i.e., sense of being in the virtual rather than in the real, physical environment) while driving a PW in the miWe-CC. METHOD: A qualitative research design with structured interviews was used. Fifteen clinicians and 11 children were interviewed after driving a power wheelchair (PW) in the miWe-CC simulator. Data were analyzed using the conventional and directed content analysis approaches. RESULTS: Overall, participants enjoyed using the simulator and experienced a sense of presence in the virtual space. They felt a sense of being in the virtual environment, involved and focused on driving the virtual PW rather than on the surroundings of the actual room where they were. Participants reported several similarities between the virtual community centre layout and activities of the miWe-CC and the day-to-day reality of paediatric PW users. CONCLUSION: The simulator replicated participants' expectations of real-life PW use and promises to have an effect on improving the driving skills of new PW users. Implications for rehabilitation Among young users, the McGill immersive wheelchair (miWe) simulator provides an experience of presence within the virtual environment. This experience of presence is generated by a sense of being in the virtual scene, a sense of being involved, engaged, and focused on interacting within the virtual environment, and by the perception that the virtual environment is consistent with the real world. The miWe is a relevant and accessible approach, complementary to real world power wheelchair training for young users.

Construct validity of the family impact of assistive technology scale for augmentative and alternative communication.

The Family Impact of Assistive Technology Scale for Augmentative and Alternative Communication (FIATS-AAC) measures parent-reported functioning and factors that affect functioning in children who use AAC. The aim of the current study was to assess its construct validity. For the study, 47 parents of children with AAC needs completed the FIATS-AAC and two other parent-reported questionnaires: a child quality-of-life measure and a community participation measure. An interview was also conducted with a sub-set of six parents. The FIATS-AAC showed a significant correlation with the child quality-of-life measure, but no significant associations with the community participation measure. Interviews suggested some consistency between parents' perceptions of their child's communicative functioning after comparing their FIATS-AAC scores and interview responses. This study provides evidence for the emerging construct validity of the FIATS-AAC as a measure linked to psychosocial aspects of quality-of-life in children with AAC needs between the ages of 6- and 12-years.

Responsiveness of a parent-reported outcome measure to evaluate AAC interventions for children and youth with complex communication needs.

Evaluation of the effectiveness of augmentative and alternative communication (AAC) interventions requires reliable measures that are responsive to change. The aim of this study was to explore the potential of the Family Impact of Assistive Technology Scale for AAC (FIATS-AAC) to detect functional change in children and youth with AAC needs, aged 3-17 years, and their families, 6 and 12 weeks after receiving a graphic-based, speech-generating device (SGD). Parents whose children were awaiting a SGD as part of their regular AAC service participated in the study. In all, 45 parents completed the FIATS-AAC during each of three phone interviews: at the time of device delivery, and then 6 weeks and 12 weeks after receiving the device. Children and youth were aged 3-16 years (M = 7.8, SD = 3.3) and were mostly context-dependent communicators. Paired t-tests indicated statistically significant gains in functioning from baseline to both 6 and 12 weeks after receiving the AAC device. Effect sizes were 0.41 and 0.38, respectively. This study provides initial support for the ability of the FIATS-AAC to detect functional changes in children and youth and their families after receiving a graphic-based SGD.

Exploring the functional impact of adaptive seating on the lives of individual children and their families: a collective case study.

PURPOSE: This study examined parent-reported change in the functional performance of four school-aged children with wheeled mobility needs who had used a new adaptive seating system for 6 weeks. METHODS: The collective case study involved four mothers whose children, ages 6-9 years, received a new adaptive seating system for a manual wheelchair or stroller. Mothers completed the Family Impact of Assistive Technology Scale for Adaptive Seating (FIATS-AS) at the time their child received a new seating system, and then after 6 weeks of daily use. Other questionnaires, health records, and semi-structured interviews provided additional data about the seating interventions and their functional effects on individual children and their families. RESULTS: The FIATS-AS detected overall functional gain in one family, and both gains and losses in 2-7 dimensions for all families. Functional status and change scores showed consistency with measures of seating intervention satisfaction, global functional change, and home participation. Interview themes also suggested consistency with change scores, but provided a deeper understanding of important factors that influenced adaptive seating outcomes. CONCLUSION: This study supports the need to explore further the complexity, temporality and meaningfulness of adaptive seating outcomes in individual children and their families. Implications for Rehabilitation Assistive technology practitioners need to adopt practical measurement strategies that consider the complexity, temporality, and meaningfulness of outcomes to make evidence-informed decisions about how to improve adaptive seating services and interventions. Health measurement scales that measure adaptive seating outcomes for service applications must have adequate levels of reliability and validity, as well as demonstrate responsive to important change over time for individual children and their families. Needs-specific measurement scales provide a promising avenue for understanding functional outcomes for individual children and youth who use adaptive seating systems.

Splenosis involving the gastric fundus, a rare cause of massive upper gastrointestinal bleeding: a case report and review of the literature.

Splenosis, the autotransplantation of splenic tissue following splenic trauma, is uncommonly clinically significant. Splenosis is typically diagnosed incidentally on imaging or at laparotomy and has been mistakenly attributed to various malignancies and pathological conditions. On the rare occasion when splenosis plays a causative role in a pathological condition, a diagnostic challenge may ensue that can lead to a delay in both diagnosis and treatment. The following case report describes a patient presenting with a massive upper gastrointestinal bleed resulting from arterial enlargement within the gastric fundus secondary to perigastric splenosis. The cause of the bleeding was initially elusive and this case highlights the importance of a thorough clinical history when faced with a diagnostic challenge. Treatment options, including the successful use of transarterial embolization in this case, are also presented.

Lessons learned from studying the functional impact of adaptive seating interventions for children with cerebral palsy.

Little empirical evidence exists about the effectiveness of assistive technology interventions for children with cerebral palsy (CP) to inform clinical practice. This article reviews what we know about the functional impact of adaptive seating interventions - a common assistive technology type recommended for children with CP. A contemporary assistive technology outcomes framework is considered as a way to model the temporality and measure the effects of seating interventions and moderating cofactors. Three research studies are profiled to illustrate different research methods, measurement approaches, and follow-up periods to learn about adaptive seating outcomes. Recommendations for future research include the adoption of common measurement indicators, consideration of quality assessment criteria, and the use of varied methodologies to generate new knowledge about functional outcomes. It is suggested that the proposed strategies will lead to new understandings, clinical applications, and ultimately improvements in the everyday lives of children with CP and their families.

Exploring suitable participation tools for children who need or use power mobility: A modified Delphi survey.

OBJECTIVES: To identify suitable tools for measuring important elements of participation for children, aged 18 months to 12 years, who need or use power mobility, and to indicate which tools should be considered for inclusion in a measurement toolkit. METHODS: Parents, therapists and researchers with expertise in paediatric power mobility and participation (n = 70) completed an online modified Delphi survey, with consensus set a priori >80% agreement. Existing tools were matched against participation elements ranked most important for those in early childhood (18 months-5 years) and of school-age (6-12 years) by the panel. RESULTS: Six out of 13 tools demonstrated potential, meeting at least three elements each, although none addressed all elements deemed important to measure by the panel. Only the Participation and Environment Measure for Children and Youth (PEM-CY) reached consensus for inclusion in a participation measure toolkit. CONCLUSION: Further evaluation of these tools with this population is warranted.

Measuring Participation for Children and Youth With Power Mobility Needs: A Systematic Review of Potential Health Measurement Tools.

OBJECTIVES: To identify and critically appraise potential participation measurement tools for children aged 18 months to 17 years with power mobility (PM) needs. DATA SOURCES: Searches in 9 electronic databases identified peer-reviewed publications in English to January 2015, along with hand-searching included bibliographies. STUDY SELECTION: The Preferred Reporting Items for Systematic reviews and Meta-Analyses statement was followed with inclusion criteria set a priori. Keywords and subject headings included participation and measurement terms with descriptors of young people who are potential PM candidates. Publications describing measurement properties of English-language tools were included if the items included ≥ 85% content related to participation and described at least 2 participation dimensions. DATA EXTRACTION: Two reviewers reached consensus after independently screening titles and abstracts, identifying full-text articles meeting criteria, extracting data, and conducting quality ratings. Tool descriptions, clinical utility, and measurement properties were extracted. Study quality and measurement properties were evaluated using the COnsensus-based Standards for the selection of health Measurement INstruments checklist and the McMaster Outcome Measures Rating Form. DATA SYNTHESIS: Of 1330 titles identified, 138 peer-reviewed publications met study inclusion criteria. Fifty tools were identified, of which 20 met inclusion criteria. Evidence supporting reliability and validity varied considerably. Two tools had responsiveness evidence, an important measurement property when evaluating change. Quality ratings were strongest for internal consistency and content validity. Ratings were downgraded because of small sample sizes and a limited description of missing data or study conditions. CONCLUSIONS: While potential tools emerged (Assessment of Preschool Children's Participation, Preferences for Activities of Children, Child and Adolescent Scale of Participation, Child Engagement in Daily Life, Canadian Occupational Performance Measure, Questionnaire of Young People's Participation), none were judged best suited for use with children having PM needs. Further empirical studies with this population are needed before recommending use for PM applications.

RESURGENCE OF MANDS FOLLOWING FUNCTIONAL COMMUNICATION TRAINING.

Experimental conditions similar to those described by Lieving and Lattal (2003) were used within two experiments to evaluate the resurgence of mands with humans. Two mands from the same operant class were trained with three participants with developmental disabilities during Experiment 1 and with two participants with developmental disabilities and a history of problem behavior during Experiment 2. The two mands were then placed on extinction. Both persisted, but showed different response strength during extinction. The mand with the weaker response strength was targeted for additional functional communication training and the alternative mand was placed on extinction. Following steady levels of occurrence of the targeted mand and no occurrences of the alternative mand, both mands were placed on extinction again. At least one instance of resurgence of the alternative mand occurred with every participant and resurgence of problem behavior occurred for both participants during Experiment 2.

Randomized Trial Comparing the Primary Patency following Cutting Versus High-Pressure Balloon Angioplasty for Treatment of de Novo Venous Stenoses in Hemodialysis Arteriovenous Fistulae.

PURPOSE: A single-center randomized clinical trial was performed to compare postinterventional primary patency rates achieved by cutting balloon angioplasty and high-pressure balloon angioplasty in the treatment of de novo stenoses within autogenous arteriovenous (AV) fistulae for hemodialysis. MATERIALS AND METHODS: Forty-eight patients undergoing their first angioplasty were prospectively randomized to undergo angioplasty with a cutting balloon or high-pressure balloon 4-8 mm in diameter because cutting balloons larger than 8 mm are not available. Nine patients were excluded after angiography, with seven requiring balloons larger than 8 mm. In the remaining 39 patients, there were 42 stenoses in the following regions: juxtaanastomotic (38%), perianstomotic (38%), midcephalic (9%), and cephalic arch (14%). Patients in the cutting balloon group were younger (mean age difference, 9 y; P = .04), but other demographic variables were comparable (range, P = .08-.89). The mean follow-up period was 8.5 mo (range, 24 d to 32 mo). Kaplan-Meier analysis was used to compare duration of patency. Mann-Whitney rank-sum t test and χ2/Fisher exact tests were used to compare continuous and categoric variables, respectively. RESULTS: Technical success was achieved in all 39 patients. At 3, 6, and 12 months, the postinterventional primary patency rates for the cutting balloon group were 61.1% (95% confidence interval [CI], 35.75%-82.70%), 27.7% (95% CI, 9.69%-53.48%), and 11.1% (95% CI, 1.38%-34.71%), respectively, compared with 70.0% (95% CI, 45.72%-88.11%), 42.1% (95% CI, 20.25%-66.50%), and 26.3% (95% CI, 9.15%-51.20%), respectively, for the high-pressure balloon group (P < .3 at each interval). CONCLUSIONS: Compared with high-pressure balloon angioplasty, cutting balloon angioplasty does not improve postinterventional primary patency of de novo stenotic lesions in autogenous arteriovenous fistulae.

Towards Advancing Knowledge Translation of AAC Outcomes Research for Children and Youth with Complex Communication Needs.

The production of new knowledge in augmentative and alternative communication (AAC) requires effective processes to leverage the different perspectives of researchers and knowledge users and improve prospects for utilization in clinical settings. This article describes the motivation, planning, process, and outcomes for a novel knowledge translation workshop designed to influence future directions for AAC outcomes research for children with complex communication needs. Invited knowledge users from 20 pediatric AAC clinics and researchers engaged in the collaborative development of research questions using a framework designed for the AAC field. The event yielded recommendations for research and development priorities that extend from the early development of language, communication, and literacy skills in very young children, to novel but unproven strategies that may advance outcomes in transitioning to adulthood.

Ethics in pharmacologic research in the child with a disability.

This article reviews the unique ethical concerns that face clinicians, researchers, and family members in the realm of research involving children and youth with childhood-onset disabilities. Presented are the contemporary legal and regulatory environments in which we work and a synopsis of relevant articles on bioethics in this sector of the scientific literature. The most important ethical themes that emerged for children with disabilities include justice in research, consent and assent, child-centered communication, child- and family-centered decision making, participation in multiple studies, and therapeutic misconception. Two publicly recorded clinical studies are profiled to illustrate common considerations and concerns that arise during our ethical review of drug studies involving children with disabilities. It is concluded that the balance of access to current research and treatments must be weighed against risk for all involved. Collaborative planning amongst those involved in the development, review, approval, conduct, and oversight of drug study protocols can lead to effective scientific inquiry within the context of core ethical principles and child- and family-centered care.

Important elements of measuring participation for children who need or use power mobility: a modified Delphi survey.

AIM: To identify and reach consensus on important elements of measuring participation in everyday life for children who need or use power mobility. METHOD: A panel (n=74) of parents, therapists, and researchers with pediatric power mobility and participation expertise completed an online modified Delphi survey. Three rounds determined important elements of participation for two groups: early childhood (18mo-5y) and school-aged (6-12y). 'Elements of participation' defined the 'who, what, where, and how' of measuring participation, generated from a literature review and participants' suggestions. Consensus was set a priori as ≥80% agreement. RESULTS: Consensus was reached on 21 out of 48 elements of participation important to measure for our population: eight elements for the younger group and 18 elements for the older group. When ranked by importance, four of the top five elements were common across both age groups. INTERPRETATION: For children using power mobility, measuring participation in a variety of settings is critical, along with considering both the child's and family's participation. Evaluating child engagement and enjoyment of participation are priorities, as is measuring barriers and facilitators. For school-aged children, evaluating child and parent reports of participation are essential. These elements can guide tool selection and/or development.